Early recognition and treatment of laryngeal swelling is essential owing to the potential life-threatening upper airway obstruction. Moreover, chronic laryngeal swelling may be caused by malignant or inflammatory conditions, such as amyloidosis,1 sarcoidosis,2 or IgG4-related disease.3 Here, we report a case with persistent laryngeal swelling caused by primary intralymphatic histiocytosis (ILH), a very rare benign disease that usually presents cutaneously.4,5